Abstract

The association between thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) is rare. Herein, we present a case with overlapping features between TTP and SLE. A 17-year old girl admitted to our hospital with an initial complaint of high grade fever, persistent headache, and microangiopathic hemolytic anaemia (negative Coombs test with schistocytes), thrombocytopenia with thrombotic event with central nervous system involvement and probably renal involvement. Also, our patient fulfilled the criteria for SLE- fever, hair loss, oral ulcers, central nervous system involvement, renal involvement, positive ANA, positive dsDNA and normal C3 and C4. The patient was rescued by extensive plasma exchange started promptly after the diagnosis. After 8 months of treatment, TTP recurred, successfully managed with plasma exchange, steroids and cyclophosphamide. It was difficult to discriminate TTP from SLE. Our case presented simultaneous features of both SLE and TTP who reinforce the importance of early diagnosis of TTP by the observation of fragmented RBCs and the intensive therapy, including plasma exchange and careful follow-up, not only to ensure diagnosis and treatment of a relapsed episode of TTP, but also to ensure proper management of the patient’s physical and mental health due to its high morbidity.